Plp1

Summary

Gene Symbol: Plp1
Description: proteolipid protein (myelin) 1
Alias: DM20, Plp, jimpy, msd, rsh, myelin proteolipid protein, lipophilin, myelin synthesis deficiency, rump shaker
Species: mouse
Products:     Plp1

Top Publications

  1. Thomson C, Anderson T, McCulloch M, Dickinson P, Vouyiouklis D, Griffiths I. The early phenotype associated with the jimpy mutation of the proteolipid protein gene. J Neurocytol. 1999;28:207-21 pubmed
    The jimpy mutation of the X-linked proteolipid protein (Plp) gene causes dysmyelination and premature death of the mice...
  2. Billings Gagliardi S, Nunnari J, Nadon N, Wolf M. Evidence that CNS hypomyelination does not cause death of jimpy-msd mutant mice. Dev Neurosci. 1999;21:473-82 pubmed
    Mice expressing three of the proteolipid protein (Plp) mutations in the mouse (jimpy, jimpy-msd, and jimpy-4J) all have a severe deficiency of CNS myelin and oligodendrocytes (OLs), and die sometime in their 4th postnatal week...
  3. Wang E, Dimova N, Sperle K, Huang Z, Lock L, McCulloch M, et al. Deletion of a splicing enhancer disrupts PLP1/DM20 ratio and myelin stability. Exp Neurol. 2008;214:322-30 pubmed publisher
    b>PLP1 and DM20, major myelin proteins, are generated by developmentally regulated alternative splicing. In the post-natal brain, PLP1 is the predominant product...
  4. Readhead C, Schneider A, Griffiths I, Nave K. Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage. Neuron. 1994;12:583-95 pubmed
    ..Mutations of the X chromosome-linked PLP gene cause glial cell death and myelin deficiency in jimpy mice and other neurological mutants...
  5. Pereira G, Dobretsova A, Hamdan H, Wight P. Expression of myelin genes: comparative analysis of Oli-neu and N20.1 oligodendroglial cell lines. J Neurosci Res. 2011;89:1070-8 pubmed publisher
    ..Analysis of the splice isoforms expressed by the myelin proteolipid protein (Plp1), myelin basic protein (Mbp), and 2',3'-cyclic nucleotide 3'-phosphodiesterase (Cnp) genes, along ..
  6. Fancy S, Baranzini S, Zhao C, Yuk D, Irvine K, Kaing S, et al. Dysregulation of the Wnt pathway inhibits timely myelination and remyelination in the mammalian CNS. Genes Dev. 2009;23:1571-85 pubmed publisher
    ..Evidence of Wnt pathway activity in human MS lesions suggests that its dysregulation might contribute to inefficient myelin repair in human neurological disorders. ..
  7. Timsit S, Bally Cuif L, Colman D, Zalc B. DM-20 mRNA is expressed during the embryonic development of the nervous system of the mouse. J Neurochem. 1992;58:1172-5 pubmed
    ..the polymerase chain reaction (PCR) and in situ hybridization to search for the presence of proteolipid protein (PLP) gene transcripts in the developing mouse...
  8. Skoff R, Ghandour M. Oligodendrocytes in female carriers of the jimpy gene make more myelin than normal oligodendrocytes. J Comp Neurol. 1995;355:124-33 pubmed
    The female carrier of the jimpy (jp) gene is a model system to study the plasticity of neuroglial cells and the mechanisms they use to compensate for a temporary deficit in myelin...
  9. Bongarzone E, Campagnoni C, Kampf K, Jacobs E, Handley V, Schonmann V, et al. Identification of a new exon in the myelin proteolipid protein gene encoding novel protein isoforms that are restricted to the somata of oligodendrocytes and neurons. J Neurosci. 1999;19:8349-57 pubmed
    The myelin proteolipid protein (PLP) gene (i.e., the PLP/DM20 gene) has been of some interest because of its role in certain human demyelinating diseases, such as Pelizaeus-Merzbacher disease...

More Information

Publications62

  1. Li S, Moore C, Dobretsova A, Wight P. Myelin proteolipid protein (Plp) intron 1 DNA is required to temporally regulate Plp gene expression in the brain. J Neurochem. 2002;83:193-201 pubmed
    The myelin proteolipid protein (Plp) gene encodes the most abundant protein found in mature CNS myelin...
  2. Al Saktawi K, McLaughlin M, Klugmann M, Schneider A, Barrie J, McCulloch M, et al. Genetic background determines phenotypic severity of the Plp rumpshaker mutation. J Neurosci Res. 2003;72:12-24 pubmed
    The rumpshaker mutation of the proteolipid protein (Plp) gene causes dysmyelination in man and mouse. We show that the phenotype in the mouse depends critically on the genetic background in which the mutation is expressed...
  3. Nadon N, Arnheiter H, Hudson L. A combination of PLP and DM20 transgenes promotes partial myelination in the jimpy mouse. J Neurochem. 1994;63:822-33 pubmed
    Mutations in the myelin proteolipid protein (PLP) gene, such as that found in the jimpy mouse, result in an abnormal structure of the myelin, severe dysmyelination, and a reduction in the number of mature oligodendrocytes...
  4. Edgar J, McLaughlin M, Werner H, McCulloch M, Barrie J, Brown A, et al. Early ultrastructural defects of axons and axon-glia junctions in mice lacking expression of Cnp1. Glia. 2009;57:1815-24 pubmed publisher
    ..2',3'-cyclic nucleotide 3'-phosphodiesterase (CNP) and in mice deficient in both CNP and proteolipid protein (PLP/DM20)...
  5. Miller M, Kangas C, Macklin W. Neuronal expression of the proteolipid protein gene in the medulla of the mouse. J Neurosci Res. 2009;87:2842-53 pubmed publisher
    The proteolipid protein (PLP) gene (Plp) encodes the major myelin proteins, PLP and DM20. Expression of Plp occurs predominantly in oligodendrocytes, but evidence is accumulating that this gene is also expressed in neurons...
  6. Jacobs E, Bongarzone E, Campagnoni C, Kampf K, Campagnoni A. Soma-restricted products of the myelin proteolipid gene are expressed primarily in neurons in the developing mouse nervous system. Dev Neurosci. 2003;25:96-104 pubmed
    The myelin proteolipid gene encodes two sets of proteins, the classic PLP and DM20 and the sr (soma-restricted)-PLP and sr-DM20...
  7. Li S, Dobretsova A, Kokorina N, Wight P. Repression of myelin proteolipid protein gene expression is mediated through both general and cell type-specific negative regulatory elements in nonexpressing cells. J Neurochem. 2002;82:159-71 pubmed
    The myelin proteolipid protein gene (Plp ) is expressed primarily in oligodendrocytes...
  8. Inoue Y, Kagawa T, Matsumura Y, Ikenaka K, Mikoshiba K. Cell death of oligodendrocytes or demyelination induced by overexpression of proteolipid protein depending on expressed gene dosage. Neurosci Res. 1996;25:161-72 pubmed
    The transgenic mice, which were produced by introducing the wild type proteolipid protein (PLP) gene, revealed different neurological symptoms depending on expressed gene dosage...
  9. Ikenaka K, Kagawa T, Mikoshiba K. Selective expression of DM-20, an alternatively spliced myelin proteolipid protein gene product, in developing nervous system and in nonglial cells. J Neurochem. 1992;58:2248-53 pubmed
    Mutations within the gene for myelin proteolipid protein (PLP), a major myelin structural protein, result in abnormal glial differentiation, suggesting that the PLP gene products play some other functional roles...
  10. Wang S, Dulin J, Wu H, Hurlock E, Lee S, Jansson K, et al. An oligodendrocyte-specific zinc-finger transcription regulator cooperates with Olig2 to promote oligodendrocyte differentiation. Development. 2006;133:3389-98 pubmed
    ..Its expression increases in parallel with that of major myelin genes Mbp and Plp1. Zfp488 is a nuclear protein that possesses transcriptional repression activity...
  11. Yoshida M, Colman D. Parallel evolution and coexpression of the proteolipid proteins and protein zero in vertebrate myelin. Neuron. 1996;16:1115-26 pubmed
    ..In conclusion, P0 and the proteolipid proteins are evolving in parallel in myelinating cells of most vertebrate species. ..
  12. Rosenbluth J, Nave K, Mierzwa A, Schiff R. Subtle myelin defects in PLP-null mice. Glia. 2006;54:172-82 pubmed
    This study explores subtle defects in the myelin of proteolipid protein (PLP)-null mice that could potentially underlie the functional losses and axon damage known to occur in this mutant and in myelin diseases including multiple ..
  13. Vouyiouklis D, Barrie J, Griffiths I, Thomson C. A proteolipid protein-specific pre-mRNA (Ppm-1) contains intron 3 and is up-regulated during myelination in the CNS. J Neurochem. 2000;74:940-8 pubmed
    ..first expressed during active myelination, and it localises to the nucleus of oligodendrocytes, in both normal and jimpy (jp) murine CNS. In addition to mouse, Ppm-1 is found also in rat and dog, but not toad or trout...
  14. Michalski J, Anderson C, Beauvais A, De Repentigny Y, Kothary R. The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development. PLoS ONE. 2011;6:e19772 pubmed publisher
    The proteolipid protein (Plp) gene promoter is responsible for driving expression of one of the major components of myelin--PLP and its splice variant DM-20...
  15. Dickinson P, Fanarraga M, Griffiths I, Barrie J, Kyriakides E, Montague P. Oligodendrocyte progenitors in the embryonic spinal cord express DM-20. Neuropathol Appl Neurobiol. 1996;22:188-98 pubmed
    ..Transcripts for the dm-20 isoform of the proteolipid protein (plp) gene are detectable initially in cells of the ventral ventricular region of the embryonic central canal and ..
  16. Wight P, Dobretsova A, Macklin W. Regulation of murine myelin proteolipid protein gene expression. J Neurosci Res. 1997;50:917-27 pubmed
    To identify putative sequences that direct cell type-specific expression and/or enhance proteolipid protein (PLP) gene expression, glial or nonglial cells were transfected with various PLP-luciferase constructs that collectively span the ..
  17. Nave K, Lai C, Bloom F, Milner R. Splice site selection in the proteolipid protein (PLP) gene transcript and primary structure of the DM-20 protein of central nervous system myelin. Proc Natl Acad Sci U S A. 1987;84:5665-9 pubmed
    Proteolipid protein (PLP) is the major myelin membrane protein of the central nervous system...
  18. Stolt C, Lommes P, Friedrich R, Wegner M. Transcription factors Sox8 and Sox10 perform non-equivalent roles during oligodendrocyte development despite functional redundancy. Development. 2004;131:2349-58 pubmed
    ..Resulting differences in protein amounts might be a main reason for the weaker impact of Sox8 on oligodendrocyte development and for unidirectional compensation of the Sox8 loss by Sox10. ..
  19. Ye F, Chen Y, Hoang T, Montgomery R, Zhao X, Bu H, et al. HDAC1 and HDAC2 regulate oligodendrocyte differentiation by disrupting the beta-catenin-TCF interaction. Nat Neurosci. 2009;12:829-38 pubmed publisher
    ..Thus, crosstalk between HDAC1/2 and the canonical Wnt signaling pathway mediated by TCF7L2 serves as a regulatory mechanism for oligodendrocyte differentiation. ..
  20. Fanarraga M, Sommer I, Griffiths I, Montague P, Groome N, Nave K, et al. Oligodendrocyte development and differentiation in the rumpshaker mutation. Glia. 1993;9:146-56 pubmed
    The jimpy rumpshaker (jprsh) mutation is an amino acid substitution in exon 4 (Ile186-->Thr) of the proteolipid protein (PLP) gene on the X chromosome...
  21. Schneider A, Griffiths I, Readhead C, Nave K. Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein. Proc Natl Acad Sci U S A. 1995;92:4447-51 pubmed
    ..A point mutation in the gene for myelin proteolipid protein (PLP) underlies oligodendrocyte death and dysmyelination in jimpy mice, an accurate model for Pelizaeus-..
  22. Stolt C, Rehberg S, Ader M, Lommes P, Riethmacher D, Schachner M, et al. Terminal differentiation of myelin-forming oligodendrocytes depends on the transcription factor Sox10. Genes Dev. 2002;16:165-70 pubmed
    ..Sox10 directly regulates myelin gene expression in oligodendrocytes, but does not control erbB3 expression as in peripheral glia. Sox10 thus functions in peripheral and central glia at different stages and through different mechanisms. ..
  23. Boison D, Bussow H, D Urso D, Muller H, Stoffel W. Adhesive properties of proteolipid protein are responsible for the compaction of CNS myelin sheaths. J Neurosci. 1995;15:5502-13 pubmed
    We have studied the molecular function of proteolipid protein (PLP), the main integral membrane protein of CNS myelin, by generating mice lacking PLP expression...
  24. Finzsch M, Stolt C, Lommes P, Wegner M. Sox9 and Sox10 influence survival and migration of oligodendrocyte precursors in the spinal cord by regulating PDGF receptor alpha expression. Development. 2008;135:637-46 pubmed publisher
    ..We thus conclude that Sox9 and Sox10 are required in a functionally redundant manner in oligodendrocyte precursors for PDGF-dependent survival and migration. ..
  25. Kagawa T, Ikenaka K, Inoue Y, Kuriyama S, Tsujii T, Nakao J, et al. Glial cell degeneration and hypomyelination caused by overexpression of myelin proteolipid protein gene. Neuron. 1994;13:427-42 pubmed
    b>Myelin proteolipid protein (PLP), the major myelin protein in the CNS, has been thought to function in myelin assembly...
  26. Anderson T, Klugmann M, Thomson C, Schneider A, Readhead C, Nave K, et al. Distinct phenotypes associated with increasing dosage of the PLP gene: implications for CMT1A due to PMP22 gene duplication. Ann N Y Acad Sci. 1999;883:234-46 pubmed
    Increased dosage of the proteolipid protein (Plp) gene causes CNS disease (Pelizaeus-Merzbacher disease [PMD]), which has many similarities to disorders of the PNS associated with duplication of the peripheral myelin protein-22 (PMP22) ..
  27. Lu Q, Sun T, Zhu Z, Ma N, Garcia M, Stiles C, et al. Common developmental requirement for Olig function indicates a motor neuron/oligodendrocyte connection. Cell. 2002;109:75-86 pubmed
    ..Neither Olig gene is required for astrocytes. These findings, together with fate mapping analysis of Olig-expressing cells, indicate that oligodendrocytes are derived from Olig-specified progenitors that give rise also to neurons. ..
  28. Spassky N, Goujet Zalc C, Parmantier E, Olivier C, Martinez S, Ivanova A, et al. Multiple restricted origin of oligodendrocytes. J Neurosci. 1998;18:8331-43 pubmed
    The plp gene encodes the proteolipid protein and its alternatively spliced product DM-20, major proteins of CNS myelin. In the mouse, plp/dm-20 transcripts are expressed beginning at embryonic day 9.5 (E9...
  29. Campagnoni C, Garbay B, Micevych P, Pribyl T, Kampf K, Handley V, et al. DM20 mRNA splice product of the myelin proteolipid protein gene is expressed in the murine heart. J Neurosci Res. 1992;33:148-55 pubmed
    ..Northern analysis also revealed the expression of the DM20 mRNA in the hearts of the jimpy and quaking mutants...
  30. Garbern J, Yool D, Moore G, Wilds I, Faulk M, Klugmann M, et al. Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination and inflammation. Brain. 2002;125:551-61 pubmed
    ..of rodents and humans with a null mutation in the gene encoding the major CNS myelin protein, proteolipid protein (PLP1, previously PLP)...
  31. Macklin W, Campagnoni C, Deininger P, Gardinier M. Structure and expression of the mouse myelin proteolipid protein gene. J Neurosci Res. 1987;18:383-94 pubmed
    The gene for the mouse myelin proteolipid protein has been isolated and the seven exons have been sequenced...
  32. Griffiths I, Klugmann M, Anderson T, Yool D, Thomson C, Schwab M, et al. Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. Science. 1998;280:1610-3 pubmed
    ..Two myelin membrane proteolipids, PLP and DM20, were shown to be essential for the integrity of myelinated axons...
  33. Griffiths I, Scott I, McCulloch M, Barrie J, McPhilemy K, Cattanach B. Rumpshaker mouse: a new X-linked mutation affecting myelination: evidence for a defect in PLP expression. J Neurocytol. 1990;19:273-83 pubmed
    ..Genetic analysis indicates a locus at or close to the PLP/jimpy (jp) locus...
  34. Kagawa T, Nakao J, Yamada M, Shimizu K, Hayakawa T, Mikoshiba K, et al. Fate of jimpy-type oligodendrocytes in jimpy heterozygote. J Neurochem. 1994;62:1887-93 pubmed
    In the jimpy mutant mouse, as well as in many other animals with mutations in the myelin proteolipid protein (PLP) gene, oligodendrocytes degenerate before their maturation...
  35. Qi Y, Cai J, Wu Y, Wu R, Lee J, Fu H, et al. Control of oligodendrocyte differentiation by the Nkx2.2 homeodomain transcription factor. Development. 2001;128:2723-33 pubmed
    ..2 is also expressed in mammalian oligodendrocyte progenitors and that the differentiation of MBP-positive and PLP-DM20-positive oligodendrocytes is dramatically retarded in Nkx2.2-null mutants along the entire rostrocaudal axis...
  36. Stecca B, Southwood C, Gragerov A, Kelley K, Friedrich V, Gow A. The evolution of lipophilin genes from invertebrates to tetrapods: DM-20 cannot replace proteolipid protein in CNS myelin. J Neurosci. 2000;20:4002-10 pubmed
    ..gene encodes two myelin-specific protein isoforms, DM-20 and PLP, which are members of the highly conserved lipophilin family of transmembrane proteins...
  37. Nave K. Neurological mouse mutants and the genes of myelin. J Neurosci Res. 1994;38:607-12 pubmed
    ..Shiverer, jimpy, Trembler, and protein zero (P0)-deficient mice demonstrate the overall function of myelination and have become ..
  38. Lu Q, Yuk D, Alberta J, Zhu Z, Pawlitzky I, Chan J, et al. Sonic hedgehog--regulated oligodendrocyte lineage genes encoding bHLH proteins in the mammalian central nervous system. Neuron. 2000;25:317-29 pubmed
    ..Gain- and loss-of-function analyses in transgenic mice demonstrate that Shh is both necessary and sufficient for Olg gene expression in vivo. ..
  39. Komaki H, Sasaki M, Yamamoto T, Iai M, Takashima S. Connatal Pelizaeus-Merzbacher disease associated with the jimpy(msd) mice mutation. Pediatr Neurol. 1999;20:309-11 pubmed
    ..In a patient with connatal Pelizaeus-Merzbacher disease with the same mutation in the proteolipid protein gene as in jimpy(msd) mice the immunohistochemical study of the brain demonstrated deficiencies of myelin and proteolipid protein ..
  40. Klugmann M, Schwab M, Pühlhofer A, Schneider A, Zimmermann F, Griffiths I, et al. Assembly of CNS myelin in the absence of proteolipid protein. Neuron. 1997;18:59-70 pubmed
    Two proteolipid proteins, PLP and DM20, are the major membrane components of central nervous system (CNS) myelin. Mutations of the X-linked PLP/DM20 gene cause dysmyelination in mouse and man and result in significant mortality...
  41. Nadon N, Miller S, Draeger K, Salvaggio M. Myelin proteolipid DM20: evidence for function independent of myelination. Int J Dev Neurosci. 1997;15:285-93 pubmed
    b>DM20 is a proteolipid protein that has been extensively studied for its role in central nervous system myelination. We demonstrate that DM20 expression is widespread and independent of myelination...
  42. Stolt C, Schlierf A, Lommes P, Hillgärtner S, Werner T, Kosian T, et al. SoxD proteins influence multiple stages of oligodendrocyte development and modulate SoxE protein function. Dev Cell. 2006;11:697-709 pubmed
    ..Our studies reveal a complex regulatory network between different groups of Sox proteins that is essential for proper progression of oligodendrocyte development. ..
  43. Guo F, Maeda Y, Ma J, Xu J, Horiuchi M, Miers L, et al. Pyramidal neurons are generated from oligodendroglial progenitor cells in adult piriform cortex. J Neurosci. 2010;30:12036-49 pubmed publisher
    ..Our data suggest that NG2(+)/PDGFR?(+) proteolipid protein promoter-expressing progenitors generate pyramidal glutamatergic neurons within normal adult piriform cortex. ..
  44. Johnson R, Roder J, Riordan J. Over-expression of the DM-20 myelin proteolipid causes central nervous system demyelination in transgenic mice. J Neurochem. 1995;64:967-76 pubmed
    ..of a transgene coding for normal DM-20, the alternatively spliced quantitatively minor isoform of myelin proteolipid protein. Demyelination of the CNS occurs as a consequence of 70 copies of this transgene...
  45. Vela J, Gonzalez B, Castellano B. Understanding glial abnormalities associated with myelin deficiency in the jimpy mutant mouse. Brain Res Brain Res Rev. 1998;26:29-42 pubmed
    b>Jimpy is a shortened life-span murine mutant showing recessive sex-linked inheritance...
  46. Mitchell L, Gillespie S, McAllister F, Fanarraga M, Kirkham D, Kelly B, et al. Developmental expression of major myelin protein genes in the CNS of X-linked hypomyelinating mutant rumpshaker. J Neurosci Res. 1992;33:205-17 pubmed
    ..an X-linked mutation causing hypomyelination of the CNS of mice and has recently been identified as an allele of jimpy (jp)...
  47. Rosenbluth J, Stoffel W, Schiff R. Myelin structure in proteolipid protein (PLP)-null mouse spinal cord. J Comp Neurol. 1996;371:336-44 pubmed
    Fixed preparations of proteolipid protein (PLP)-null mouse spinal cord show myelin sheaths which in some regions consist of typical alternating major dense lines (MDLs) and intermediate lines (ILs) with a repeat period of 10.3 nm...
  48. Yool D, Edgar J, Montague P, Malcolm S. The proteolipid protein gene and myelin disorders in man and animal models. Hum Mol Genet. 2000;9:987-92 pubmed
    The two proteins, proteolipid protein and DM20, which are encoded by alternative transcripts from the proteolipid protein ( PLP ) gene, are major components of central nervous system myelin...
  49. Calver A, Hall A, Yu W, Walsh F, Heath J, Betsholtz C, et al. Oligodendrocyte population dynamics and the role of PDGF in vivo. Neuron. 1998;20:869-82 pubmed
    ..Therefore, cell survival controls override proliferation controls for determining the final number and distribution of mature oligodendrocytes. ..
  50. Gow A, Southwood C, Lazzarini R. Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease. J Cell Biol. 1998;140:925-34 pubmed
    ..PMD) is a dysmyelinating disease resulting from mutations, deletions, or duplications of the proteolipid protein (PLP) gene. Distinguishing features of PMD include pleiotropy and a range of disease severities among patients...
  51. Timsit S, Martinez S, Allinquant B, Peyron F, Puelles L, Zalc B. Oligodendrocytes originate in a restricted zone of the embryonic ventral neural tube defined by DM-20 mRNA expression. J Neurosci. 1995;15:1012-24 pubmed
    Products of the PLP gene, proteolipid protein and its isoform DM-20, are the most abundant proteins in CNS myelin, and are markers of the oligodendrocyte, the myelin-forming cell in the CNS...
  52. Edgar J, McLaughlin M, Yool D, Zhang S, Fowler J, Montague P, et al. Oligodendroglial modulation of fast axonal transport in a mouse model of hereditary spastic paraplegia. J Cell Biol. 2004;166:121-31 pubmed
    ..Using a mouse model of hereditary spastic paraplegia type 2 due to a null mutation of the myelin Plp gene, we find a progressive impairment in fast retrograde and anterograde transport...
  53. Ip C, Kroner A, Bendszus M, Leder C, Kobsar I, Fischer S, et al. Immune cells contribute to myelin degeneration and axonopathic changes in mice overexpressing proteolipid protein in oligodendrocytes. J Neurosci. 2006;26:8206-16 pubmed
    Overexpression of the major myelin protein of the CNS, proteolipid protein (PLP), leads to late-onset degeneration of myelin and pathological changes in axons...